Huntington's Disease (Chorea)

Defined

Huntington's disease (HD) is a hereditary disorder of the central nervous system.It usually develops in adulthood and can cause a widerange of symptoms affecting both men and women. Most people with HD develop signs and symptoms between the ages of 40-50,although the onset of the disease can be earlier or later in life. If the disease onset begins before age 20, the condition is called juvenile Huntington's disease. Juvenile HD often presents with different symptoms and faster disease progression.

Causes

Huntington's Disease is caused by a faulty gene on chromosome 4. The gene produces a protein called Huntingtin. HD causes movement, cognitive and psychiatric disorders with a wide spectrum of signs and symptoms. If a parent has the disease, their child has a 50% chance of inheriting the gene that causes the genetic disorder. In rare cases, about 6% of HD cases, one can develop HD without having a family history of the disease. This may be the result of a genetic mutation that could happen during the father's sperm development.

Contracted and rigid muscles that affect gait (especially in young children)

Changes in fine motor skills that might be noticeable in skills such as handwriting

Tremors or slight involuntary movements

Seizures

Complications

The time from disease onset to death in HD is usually about 10 to 30 years. Juvenile HD typically results in death in less than 15 years from onset. Eventually, individuals suffering from this disease will need help with all activities of daily living and care. In the late stages of the disease the individual's understanding of their surroundings and interactions with others will remain intact for a long time even though they will likely be confined to a bed and unable to speak.

Common causes of death include-

Pneumonia or other infections

Injuries related to falls

Complications related to the inability to swallow

Suicide

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Compiled on 11/2012 by Meredith Preston

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